Unknown Heart, Stroke & Blood NIHR-supported project Lungs & Breathing

An Open-Label Long-term Follow-up Study to Evaluate the Effects of Sotatercept When Added to Background Pulmonary Arterial Hypertension (PAH) Therapy for the Treatment of PAH

Funder NIHR (part of DHSC)
Recipient organisation NIHR Wellcome Trust Cambridge Clinical Research Facility
NIHR support Recorded as supported by this research centre
Period Mar 2025 — Ongoing

In plain English

AI plain-English summary

A new drug called sotatercept is being tested in around 700 people with pulmonary arterial hypertension (PAH) to see if it remains safe and effective over four years. PAH is a progressive, fatal disease in which the arteries in the lungs narrow, forcing the right side of the heart to work harder until it eventually fails. Even with existing treatments, patients face severe limits on physical activity and quality of life. This study follows participants who previously received either sotatercept or a placebo in earlier trials, now all receiving the active drug. The earlier PULSAR study showed that adding sotatercept to standard therapy significantly improved pulmonary vascular resistance—a key measure of disease severity. If this long-term follow-up confirms sustained benefits, sotatercept could become a standard addition to existing PAH therapies, helping patients breathe more easily and stay active for longer. The study involves regular physical exams, heart function tests, a six-minute walking test, and right heart catheterisation to measure pressure inside the heart and lungs.

View original technical description
This is a clinical trial of a new treatment called sotatercept with background Pulmonary Arterial Hypertension (PAH) therapy for the treatment of PAH. This study is following up participants with PAH previously treated with sotatercept or placebo previously on other studies to understand the long-term safety, tolerability and efficacy of the treatment. PAH is a disease causing a progressive increase in pulmonary vascular resistance (PVR), resulting in right ventricular dysfunction and ultimately failure, as well as premature death. PAH is a progressive, fatal disease that causes marked limitations in physical activity and quality of life, even when treated with approved therapies. This study is supported by data from the primary phase of the PULSAR study (Phase 2, NCT03496207), in which participants taking any approved single or combination therapy for PAH were randomised to receive additional sotatercept or placebo for 24 weeks. The PULSAR study demonstrated a statistically significant improvement in its primary endpoint, PVR. The study duration for a given participant will be approximately 4 years with a Screening Period, Treatment Period and a Follow Up Period.The size of this study will be defined by the number of participants rolling over to this study from parent sotatercept studies. Approximately 700 participants are expected to enrol in the study.Participants rolling over to the A011-12 study from a blinded parent study will begin sotatercept treatment at a dose of 0.3 mg/kg (administered subcutaneously). Participants rolling over to the A011-12 study from an unblinded parent study will continue sotatercept treatment at their current dose.The study involves procedures including physical examinations, vital signs, ECG, assessment of heart pumping rate and measurement of pressure in heart and lungs via a Right Heart Catheterisation process, Six Minute Walk Test (6MWT) and an echocardiogram. This study is sponsored by Acceleron Pharma Inc.

Researchers

Joanna Pepke-Zaba (Principal Investigator)

Related Research

Grants with similar aims, by meaning.

A Phase 3, Randomized, Double-blind, Placebo-controlled study to Evaluate Sotatercept When Added to Background Pulmonary Arterial Hypertension (PAH) Therapy in Newly Diagnosed Intermediate-and High-risk PAH Patients
A Phase 3, Randomized, Double-blind, Placebo controlled Study to Evaluate Sotatercept When Added to Background Pulmonary Arterial Hypertension (PAH) Therapy in Newly Diagnosed Intermediate- and High-risk PAH Patients- HYPERION
A Phase 3, Randomized, Double-Blind, PlaceboControlled Study to Evaluate Sotatercept When Added to Maximum Tolerated Background Therapy in Participants With Pulmonary Arterial Hypertension (PAH) World Health Organization (WHO) Functional Class (FC) III or FC IV at High Risk of Mortality
A Phase 2 Open-label Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of Sotatercept (MK-7962) in Children from 1 to Less Than 18 Years of Age With PAH on Standard of Care
A Phase 2, Multicenter, Single-blinded, Randomized Study to Evaluate the Pharmacokinetics and Safety of Sotatercept (MK-7962) Administered Using Either a Weight-based or Weight-banded Approach in Participants With Pulmonary Arterial Hypertension (PAH) on Standard of Care

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